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13 Diseases affecting erythrocytes

Microcytic/hypochromic anemia

Iron deficiency anemia

FIGURE 13–1A Severe iron deficiency anemia (peripheral blood [PB] ×500).

FIGURE 13–1B Iron deficiency anemia (PB ×1000).

FIGURE 13–1C Iron deficiency anemia (bone marrow [BM] ×1000; showing shaggy cytoplasm).

Peripheral blood:

Erythrocytes are hypochromic and microcytic; large variation in size; possible thrombocytosis

Note:

Small lymphocyte depicted for size comparison.

Bone marrow:

Erythrocyte precursors are smaller and more numerous than normal and have shaggy cytoplasm. There is nuclear cytoplasmic asynchrony, with cytoplasmic maturation lagging behind that of the nucleus.

β-thalassemia minor

β/β⁺ β/β⁰ β/δβ⁰ β/δβ^lepore

FIGURE 13–2A β-Thalassemia minor (PB ×500).

FIGURE 13–2B β-Thalassemia minor (PB ×1000). The presence of basophilic stippling (arrow) is common in thalassemia minor but not in iron deficiency anemia.

Peripheral blood:

Microcytosis, slight hypochromia, target cells, basophilic stippling

β-thalassemia major

β⁰β⁰ β⁺β⁺ β⁰β⁺ δβ^lepore/δβ^lepore

FIGURE 13–3A β-Thalassemia major (PB ×500).

FIGURE 13–3B β-Thalassemia major (PB ×1000).

Peripheral blood:

Marked variation in size and shape, numerous nucleated erythrocytes, microcytes, hypochromia, target cells, basophilic stippling, tear drop cells, schistocytes, polychromasia

Hemoglobin bart hydrops fetalis syndrome

−−/−−

Peripheral blood:

Numerous nucleated erythrocytes, marked variation in size, hypochromia, variable polychromasia, macrocytes

FIGURE 13–4A Bart hemoglobin (PB ×500).

FIGURE 13–4B Bart hemoglobin (PB ×1000).

Macrocytosis

Nonmegaloblastic

FIGURE 13–5A Macrocytic (nonmegaloblastic) (PB ×500).

FIGURE 13–5B Macrocytic (nonmegaloblastic) (PB ×1000).

Peripheral blood:

Round macrocytes, leukocyte and platelet counts usually normal

Bone marrow:

No megaloblastic changes

Associated with:

Normal newborn, liver disease, chronic alcoholism

Megaloblastic anemia

FIGURE 13–6A Megaloblastic anemia (PB ×500).

FIGURE 13–6B Megaloblastic anemia (PB ×1000).

FIGURE 13–6C Megaloblastic anemia (BM original ×500).

FIGURE 13–6D Megaloblastic anemia (BM original ×1000).

Peripheral blood:

Pancytopenia, hypersegmentation of neutrophils, oval macrocytes, Howell-Jolly bodies, nucleated erythrocytes, basophilic stippling, schistocytes, spherocytes, tear drop cells, target cells, giant platelets

Note:

Characteristic triad of abnormalities: oval macrocytes, hypersegmented neutrophils, and Howell-Jolly bodies

Bone marrow:

Hypercellular, asynchrony (trilineage) with nuclear maturation lagging behind cytoplasmic maturation, giant bands, giant metamyelocytes, hypersegmented neutrophils

Associated with:

Vitamin B₁₂ deficiency, folate deficiency, myelodysplastic syndrome

Aplastic anemia

FIGURE 13–7A Aplastic anemia (PB ×1000).

FIGURE 13–7B Aplastic anemia (BM biopsy ×1000).

Peripheral blood:

Pancytopenia, normocytic, normochromic (occasional macrocytes)

Bone marrow:

Hypocellular; lymphocytes may predominate

Associated with:

Bone marrow failure

Immune hemolytic anemia

FIGURE 13–8A Immune hemolytic anemia (PB ×500).

FIGURE 13–8B Immune hemolytic anemia (PB ×1000).

Peripheral blood:

Spherocytes, schistocytes, polychromasia, nucleated erythrocytes

Associated with:

Autoimmune, alloimmune (see also hemolytic disease of the fetus and newborn, Figure 13-9), drug-induced hemolytic anemia

Note:

Erythrocyte morphology varies with cause and severity of disease.

Hemolytic disease of the fetus and newborn

FIGURE 13–9A Hemolytic disease of the fetus and newborn (PB ×500).

FIGURE 13–9B Hemolytic disease of the fetus and newborn (PB ×1000).

Peripheral blood:

Polychromasia, increased number of nucleated erythrocytes, macrocytic/normochromic, spherocytes—more common in ABO incompatibility

Associated with:

Fetal-maternal Rh and/or ABO incompatibility

Note:

Normal newborns have some nucleated erythrocytes (see Chapter 23).

Hereditary spherocytosis

FIGURE 13–10A Hereditary spherocytosis (PB ×500).

FIGURE 13–10B Hereditary spherocytosis (PB ×1000).

Peripheral blood:

Spherocytes (variable in number), polychromasia; nucleated erythrocytes possible

Associated with:

Red cell membrane defects

Hereditary elliptocytosis

FIGURE 13–11A Hereditary elliptocytosis (PB ×500).

FIGURE 13–11B Hereditary pyropoikilocytosis—before incubation (PB ×500).

FIGURE 13–11C Hereditary pyropoikilocytosis—with incubation at 41° to 45° C for 1 hour (PB ×500).

Peripheral blood:

>25% elliptocytes, usually >60% elliptocytes; indices are normocytic, normochromic

Associated with:

Red cell membrane defects

Variants of elliptocytosis

Hemolytic

Peripheral blood:

Microelliptocytes, schistocytes, spherocytes

Associated with:

Red cell membrane defects

Pyropoikilocytosis

Peripheral blood:

Elliptocytes, schistocytes, microspherocytes (see Figure 11-4, B).

Associated with:

Red cell membrane defects

Microangiopathic hemolytic anemia

FIGURE 13–12A Microangiopathic hemolytic anemia (PB ×500).

FIGURE 13–12B Microangiopathic hemolytic anemia (PB ×1000).

Peripheral blood:

Schistocytes, spherocytes, polychromasia, nucleated erythrocytes, decreased platelet count

Associated with:

Thrombotic thrombocytopenic purpura, hemolytic uremic syndrome, HELLP syndrome (Hemolytic anemia, Elevated Liver enzymes and Low Platelet count), disseminated intravascular coagulation, hypertensive crises

Note:

The degree of morphological change correlates directly with severity of the disease.

Hemoglobin CC disease

FIGURE 13–13A Hemoglobin CC (PB ×500).

FIGURE 13–13B Hemoglobin CC (PB ×1000).

Peripheral blood:

Polychromasia, target cells, spherocytes, microcytes, intracellular and/or extracellular rod-shaped crystals possible

Associated with:

Homozygous hemoglobin C (see Figure 11-7)

Hemoglobin SS disease

FIGURE 13–14A Hemoglobin SS (PB ×500).

FIGURE 13–14B Hemoglobin SS (PB ×1000).

Peripheral blood:

Sickle cells (in crises), target cells, nucleated erythrocytes, schistocytes, Howell-Jolly bodies, basophilic stippling, Pappenheimer bodies, polychromasia, increased leukocyte count with neutrophilia, thrombocytosis

Associated with:

Homozygous hemoglobin S (see Figure 11-6)

Hemoglobin SC disease

FIGURE 13–15A Hemoglobin SC (PB 500).

FIGURE 13–15B Hemoglobin SC (PB ×1000).

Peripheral blood:

Few sickle cells, target cells, intraerythrocytic crystals; crystalline aggregates of hemoglobin SC may protrude from the erythrocyte membrane.

Associated with:

Hemoglobin SC (see Figure 11-8)

Although characteristic findings for disease states are listed, not all may be present in one patient. The most common ones are depicted.

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13 Diseases affecting erythrocytes

Microcytic/hypochromic anemia

Iron deficiency anemia

FIGURE 13–1A Severe iron deficiency anemia (peripheral blood [PB] ×500).

FIGURE 13–1B Iron deficiency anemia (PB ×1000).

FIGURE 13–1C Iron deficiency anemia (bone marrow [BM] ×1000; showing shaggy cytoplasm).

Peripheral blood:

Erythrocytes are hypochromic and microcytic; large variation in size; possible thrombocytosis

Note:

Small lymphocyte depicted for size comparison.

Bone marrow:

Erythrocyte precursors are smaller and more numerous than normal and have shaggy cytoplasm. There is nuclear cytoplasmic asynchrony, with cytoplasmic maturation lagging behind that of the nucleus.

β-thalassemia minor

β/β⁺ β/β⁰ β/δβ⁰ β/δβ^lepore

FIGURE 13–2A β-Thalassemia minor (PB ×500).

FIGURE 13–2B β-Thalassemia minor (PB ×1000). The presence of basophilic stippling (arrow) is common in thalassemia minor but not in iron deficiency anemia.

Peripheral blood:

Microcytosis, slight hypochromia, target cells, basophilic stippling

β-thalassemia major

β⁰β⁰ β⁺β⁺ β⁰β⁺ δβ^lepore/δβ^lepore

FIGURE 13–3A β-Thalassemia major (PB ×500).

FIGURE 13–3B β-Thalassemia major (PB ×1000).

Peripheral blood:

Marked variation in size and shape, numerous nucleated erythrocytes, microcytes, hypochromia, target cells, basophilic stippling, tear drop cells, schistocytes, polychromasia

Hemoglobin bart hydrops fetalis syndrome

−−/−−

Peripheral blood:

Numerous nucleated erythrocytes, marked variation in size, hypochromia, variable polychromasia, macrocytes

FIGURE 13–4A Bart hemoglobin (PB ×500).

FIGURE 13–4B Bart hemoglobin (PB ×1000).

Macrocytosis

Nonmegaloblastic

FIGURE 13–5A Macrocytic (nonmegaloblastic) (PB ×500).

FIGURE 13–5B Macrocytic (nonmegaloblastic) (PB ×1000).

Peripheral blood:

Round macrocytes, leukocyte and platelet counts usually normal

Bone marrow:

No megaloblastic changes

Associated with:

Normal newborn, liver disease, chronic alcoholism

Megaloblastic anemia

FIGURE 13–6A Megaloblastic anemia (PB ×500).

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Clinical Hematology Atlas